Monday, January 15, 2018

Week 230: Vigor

Lime (remember him?) hit the 2.5 year mark this week, and in honor of that occasion had a check-up. Lemon has never minded going to see the regular pediatrician, presumably because it is not nearly as bad or as time-consuming as a visit to the CF clinic. Lacking this frame of reference, and being 2.5, Lime acted as though he were being kidnapped. A small bribe in the form of M&M's went a long way towards soothing his troubled soul, though. At the check-up we learned that he is still, in the immortal words of his first pediatrician, "tiny but vigorous." However, he is incrementally less tiny than he use to be. Whereas at his second birthday he was in the 4th percentile, he has now jumped all the way up to the (wait for it) 11th. So, still fairly minuscule but now within the magic range of 5th-95th percentile that pediatricians find reassuring.

The only minor problem is that to get him there, I've been letting him drink whole milk all night long, to the tune of a pint a night. Well, it turns out that's not so advisable from a dental perspective. I switched him over to water, which he's taken to admirably well, except of course for the part where now he's waking up at 4 a.m. again to have pre-breakfast. One imagines there must be a point at which he will be able to consume enough calories during the day to sustain himself through the night, yes?

And then there is Lemon. I hardly want to write about it for fear of tempting the fates, but the purpose of the blog is to report the news, so here it is. Lemon is eating stuff. All kinds of stuff. Sometimes, even "a lot" of stuff. Double-digit numbers of bites of the same food. Randomly trying new things. Pasta with red sauce and Parmesan cheese ("This is really delicious, Ma."). Hamburger with ketchup. Fried cheese curds. Watermelon. Weird hummus that was not exactly the same as the hummus that we have at home. Chips with seeds in them. Crackers with cheese spread on them. It's honestly shocking.

So, what's going on? Probably several things. He's continuing to get older, and to become more aware of what his peers are up to. We've been working hard at food school, and he's acquired new skills and confidence when it comes to eating. He's been healthy for something like 3 consecutive weeks, which is the longest illness-free period we've had since school started, so maybe he's finally feeling truly 100%. I have one other idea, I have no idea how valid it is, but thanks to ursodiol, his liver is functioning normally for the first time in his life. A very common symptom of liver inflammation is: poor appetite. I do wonder if for a long time he has just not been feeling like eating because of that inflammation, and now that it's resolved, it's contributing to this new interest in actually eating things. As with so many other things about treating CF, we will never know for sure. In this particular case, though, I'm willing to live with some uncertainty. 

Monday, January 8, 2018

Week 229: Two of a kind

This week marked another milestone of sorts: four years since we moved to Madison from our long-time hometown of Boston. It's been both forever and no time at all. I had a chance over the holidays to catch up with a few of the wonderful friends that we made while we lived there. Although, ironically, none of the people that I talked to actually live in Boston anymore, there's been quite a diaspora over the last decade or so. Still, I miss all of them dearly and wish there was some way to teleport around the country to see all of them more often!

One thing I definitely noticed this week was an overall uptick in Lemon's eating. I don't know if he decided to turn over a new leaf for 2018 or what, but all of a sudden he's trying more thing and eating more of the things he tries. Still not anything that would register as the volume of, say, a normal meal, but definitely different than what he's done before. We took the kids to a restaurant (ok, it was Culvers) and he actually ate about half of a chicken tender and 2/3 of a dish of vanilla frozen custard. And sat still at the table f or more than 10 minutes in doing so. This is what progress looks like around here! He's also said that he wants to learn how to take pills and to stop doing formula. One goal at a time.

After bath one night this week, Lemon explained to Lime he wears a tube at night because he has cystic fibrosis, so he is becoming at least vaguely aware of the idea that not everyone is like him. His tone when explaining it was more along the lines of "I have an amazing superpower that you don't have that lets me wear this tube and you can't have one," so I don't think he quite knows what it all means yet, but I suppose it's better to start this journey from a positive place.

One interesting tidbit that I'll just put out here for any fellow CF parents who are reading along is that we got back another batch of lab results for Lemon, this one on his vitamin levels (people with CF have a hard time absorbing fat-soluble vitamins). Interestingly, his A and E levels were in the high end of the normal range, but his D level was at the low end of normal, even though there is vitamin D in the special CF vitamins that he takes (the same one that gives him all his A and E), and we also give him extra vitamin D on top of that. We've been giving the extra D by using concentrated vitamin D drops that we add to his formula. One of the nutritionists told us that for reasons that no one seems to understand, vitamin D and tubes seem to be a bad combination. They think, although there's no proof, that vitamin D sticks to the insides of the plastic tubing rather than going into the person's belly, so giving it through the tube is ineffective. So, now we're supposed to switch over to doing that orally. It sounds like no big deal but it means readjusting one piece of our already complicated daily routine. We'll get there.

Speaking of adjustments to our routine, here's a good way to drive a patient or caregiver insane if you are a pharmacist. Say the patient has a one-week supply of a medication left, and asks for a refill of the medication. Give them one month's worth, divided into one small bottle with a week's worth of medication, and a large one with the remaining 3 weeks. Then tell them that the small bottle actually contains medicine that will expire in a week, so they should use the small bottle first, then finish up the one-week supply that they already had at their house, and then proceed with the next 3 weeks of the refill. Why not? It's not like patients and caregivers have anything else to think about...

Monday, January 1, 2018

Week 228: Into the new

Welcome to 2018! We closed out 2017 with the last few days of Grandma and Grandpa's visit, followed by a few days at a water park in the Wisconsin Dells (aka America's water park capitol), and our annual New Year's eve party. We celebrate with the Azores to ensure bed-time compatibility.

Although last week I focused on all the positive aspects of our clinic visit, there is, of course, always one cloud in the sky: nutrition, in this case. As I'd expected based on my own observations at home, Lemon had not gained any weight since June, but had grown 1.5cm since September. So, his BMI fell from the 70th percentile to the 40th. As his nurse practitioner pointed out, 40th isn't actually all that bad (long-time readers will remember the bad old days when we were in the 5th percentile for BMI). And it's not quite accurate to say that he hasn't gained any weight since June. He has. And then has lost it again. And gained and lost and gained and lost without any ability to get ahead with all the various colds he's had since school started.

So, it is time for a new and improved nutrition plan. The plan I wanted was a plan to get more calories into a smaller volume of formula, so that when we have to cut back the volume of tube feeds due to illness we don't fall quite as far behind on the calorie count. Of course, this is not a standard plan, but it turns out if you push a bit, there is a way. We left our clinic visit with a sample canister of a product with the appealing name of Scandical, which is essentially concentrated, powdered, tasteless, odorless, soluble calories. Given that Lemon was underweight from being sick off and on for so long, I tested whether adding a few tablespoons of the powder (the equivalent of about 120 calories) to his regular overnight feed could help him get the weight back on. Short answer: yes. Within a week, he'd put on over a pound. It's amazing what a hundred or so extra calories a day will do.

The only minor hitch is that, upon requesting more Scandical, I learned that the product has been discontinued, but never fear, a similar product called Duocal is also a thing. Now I just have to figure out where to get it from and how it gets paid for, but everyone needs a good January project. I do feel mildly crazy that my son's "diet" is essentially a chemistry experiment, when I'm seeing everyone posting about how they're eating only whole foods or giving up added sugar or whatever other totally laudable New Year's resolution they've come up with. Some day. He did eat 6 Froot Loops and a slice of yellow pepper today, not bad for a sick day. Did I mention we have yet another cold/cough combination going on? Well, we do, so good thing we have this new high-calorie nutrition plan to play with.

The kids are off from school next week so at least they will have one more relatively exposure-free week to recover from whatever they have now before going back. I had some fantasy that we were going to use this winter break to teach Lemon how to swallow pills. Hah. I'm sure we'll have time some day. Maybe we can do it this spring, right around when we're going to try and potty-train Lime. That should make for a soothing month or two. I already have a sense that 2018 is going to be another eventful year.

Monday, December 25, 2017

Week 227: Gifts

We got the gift of some great news this week, after spending nearly 4 hours at our quarterly clinic visit, enhanced by pulmonary function tests, chest x-ray, and a blood draw. Thanks to all the various poking and prodding, we now know that Lemon is 1.5cm taller than he was 3 months ago, that his chest x-ray looked clear, and that he did really well on the pulmonary function test/ His iron levels are good, and most amazingly, his liver enzymes are normal. For the first time since we've been measuring them. I could hardly believe it. We've had a wonderful holiday visit with Grandma and Grandpa, and the boys have gotten so many nice things from so many generous people. But, nothing for me will top seeing so many normal test results come in at once. I hope all of you had a wonderful time celebrating whatever you celebrate, and that the coming year brings you abundant happiness and good health. See you in 2018!

Monday, December 18, 2017

Week 226: Stretch and toss

On the week of a clinic visit, I always try to put together in my mind what has happened in the 3 months since we were last seen, and try to figure out what parts of our plan need to be optimized going forward. I needn't remind most of you that the past 3 months have been a fairly unrelenting series of respiratory infections of various types and degrees of severity. Still, I feel like in spite of all that, our respiratory plan is working. We have fought off all but two of those things without any antibiotics at all, and the two times that we felt like we did need to use an antibiotic, we had Cayston on hand, deployed it, and beat things back to baseline. We've just finished a week on Cayston now and Lemon is back to respiratory baseline already, so I'm pretty sure we can stop after 2 weeks, or maybe after 10 days if our care team agrees.

If I have learned anything over 4.5 years of being a CF mama, it's that if it's not respiratory, it's nutrition. That is indeed the case now--Lemon is currently lighter than he was in June, and also taller--I'll be curious to see by how much, but I can see his ankles sticking out of the new pants I bought him for school this fall. When weight goes down and height goes up, BMI goes in the toilet, and I can see it written all over his body, every rib, every vertebra, every muscle in his back clearly visible. He's not unhealthily skinny (yet, I think), but he is definitely very thin. Thin would be OK if I didn't have this suspicion that our reserves would be called upon many more time this winter, and we don't have any right now.

So, what am I hoping to get out of this clinic visit? Well, we will get a lot of data. It's our big annual recheck of everything, which means not just the regular two-hour song and dance, but also pulmonary function tests, chest x-ray, and a blood draw. I am curious to see how Lemon's lungs are doing in light of all they've been through so far this winter, although I doubt either the PFTs or the x-rays will tell us much, because Lemon isn't reliable enough at doing the PFTs yet for the data to be really valid, and an x-ray is not exactly a highly sensitive measure of how lungs are doing. I will also be very curious to see how his liver numbers are after 3 months on ursodiol--hopefully it's working and his numbers will be within the normal range, or at least closer to it. We'll also get a vitamin D level to see if he's taking enough vitamins, and some iron measures to see if the amount we're supplementing is the right amount


I'm also hoping to continue to get some new ideas from our nutritionist on how to add calories to Lemon's formula so we can get some weight back on him. I've given up all short-term hope of Lemon actually eating enough calories to have any kind of impact on his weight. With his BMI continuing to drop, I basically just want to ram as many calories as is feasible through the tube to get some weight on him over the next couple of weeks, when he'll be home from school and away from the 18 pathogen-carrying monsters (ahem, I mean his friends) in his class. We're continuing with food school for now. I had thought about pulling the plug after today's session, because Lemon had really started to resist going. I mentioned that to his therapist, and she said that she wasn't at all surprised, that she's been pushing his abilities by offering increasingly challenging foods. And Lemon being Lemon, he doesn't want to do things he's not good at so he'd rather just not go. She still thinks he's making progress, so we'll keep at it for a while longer and see where we can get.

Assuming we survive the clinic visit, we're all looking forward to the upcoming holidays. This is the first year Lemon has been old enough to really anticipate the holidays (well, mainly all his presents). Lime still doesn't quite get the idea of presents yet, but he loves all the lights and decorations. And he still can't quite say the "k" sound (usually substituting "t"), so his pronunciation of "Christmas" cracks me up every time. Don't worry, I'm still a committed Scrooge at heart, but I do appreciate seeing the magic of the holiday season through the kids eyes.

Monday, December 11, 2017

Week 225: Troughs

Thus far, our fall and winter has been kind of like the experience of being on a small boat at sea. You get buffeted by a wave, then the wave disappears. Is it calm, or is it a trough between waves? Trough. Another wave comes and goes. Calm? Nope, another trough. Another wave, calm again. Or so it seems. Trough after all. Having played a few rounds, I'm pretty confident that the answer is pretty much always trough. Some troughs may be wider than others, just to kind of mix it up, but they are all troughs in the end.

Some may say that I jinxed our last pseudo-calm by noting how calm it was. Not so. In fact, at the time that I wrote last week's post, Lime was already beginning to cough and sneeze as a result of cold #307 that was about to make its way through our family. But, I wanted to dwell for a little while longer on that flat stretch of water. As it turns out, #307 was quite a doozy. Lime was perky enough on Tuesday morning, so I brought him to school as usual. When I picked him up, though, he was in a sorry state of affairs and his teacher said to me, "Maybe not tomorrow." So, I arranged for our former nanny to come and watch him on Wednesday morning. Of course, by Tuesday night, Lemon was coughing too, and since I had childcare arranged at home anyhow I let him stay home, too. I might point out this is the first day of school he has missed this year--quite an achievement on his part.

On Thursday, both guys were feeling pretty lousy so I kept them both home again, but on Friday they both seemed quite improved so back to school they went. Lime continued to recover, whereas Lemon's recovery sort of stalled, and he continued to look really pale. I mean even paler than usual. In spite of his color and the cough he seemed sprightly enough until yesterday morning, when the cough took a marked turn for the worse--with Lime's cough almost completely gone, Lemon's was suddenly big, wet, and productive. I did not like the sound of that at all, and Papa Bear and I agreed immediately that it was time to start Cayston again. That was definitely the right call, because 24 hours in to the course of Cayston, Lemon's cough has nearly dried up and he seems to be feeling quite a bit better. Hopefully this means we can get away with just doing 2 weeks this time.

Maybe we'll get lucky and the Cayston will help keep him healthy enough for long enough that he'll put some weight on over the next 10 days. We have our big end-of-year clinic visit coming up on the 20th and I'd really rather not bring him in with his spine and all his ribs sticking out through his skin like they are now. His appearance doesn't really tell the full story of how hard we have been trying to keep up with nutrition this winter.

For the past couple of months I've been wanting to teach Lemon to swallow pills, since everyone has been getting pretty tired of the whole enzymes with applesauce show. But, with all the puking there hasn't been a good opportunity--the last thing I want is for him to draw a connection between that and swallowing pills. He also hasn't seemed that interested, in spite of not wanting to do the applesauce anymore. But, this evening, he spontaneously mentioned to me that he wants to learn to take pills whole. So, that seems hopeful, and will give us something fun to work on over the holidays. Don't worry, in addition to learning pill swallowing, Grandma and Grandpa are coming to visit and we are going to a water park, so there will be some more traditional fun as well!

Monday, December 4, 2017

Week 224: Zippy

Every now and again, maybe about 2 weeks out of every 52 it seems, we get a week where nothing much happens. And man are those the best weeks ever. Lemon and Lime both recovered from the cold they had over Thanksgiving and stayed well for the last 10 days or so. It's amazing what 10 days of good health will do for a couple of boys. By that I mean that their energy level is such that Papa Bear and I have serious concerns as to whether our house and worldly possessions will survive the onslaught. One afternoon this week when Lemon was being particularly "energetic," we at first wondered if something was wrong. It turns out, actually, that something was right--it had been a week or so since he'd last vomited, so he was finally feeling caught up on sleep and nutrition and boy did it show.

I'm glad Lemon was full of pep for his week as "child of the week" in his class at school. He got to wear a crown every day, and have a poster with pictures of his family hung in the classroom. On Wednesday, I went in and did a science demonstration for the kids, layering corn syrup, water, and olive oil in a drinking glass. I think it went well, although thus far Lemon has declined to recount the experience for anyone. After some prodding he is willing to concede that I was in his classroom, but that is about it.

Papa Bear was out of town for a couple of days this week, so at the kids' request I took them on a drive "at night time" (ie 4:45pm) to see the holiday lights around our neighborhood. Since they almost never leave the house when it's dark, this was a profoundly thrilling experience, which we topped off by picking up some take-out food from a restaurant, another cause for high excitement. I couldn't get any traction with Lemon on the chicken satay that I got for him, but weirdly he adored the cucumber salad that came with it. In other news, his relationship with food continues to mystify everyone.

Lime appears to be going through some sort of growth spurt, and I've temporarily conceded the point that he may take a sippy cup of milk to bed with him. The old me would have not been in favor of this idea at all. The new me, on the other hand, recognizes the inherent benefit implied by not getting up to pour a glass of milk at 3 a.m. He's almost up to 25 lb, so who knows, may be by his 30 month check-up in January he will have clawed his way out of the 4th percentile for weight. His little personality is getting more assertive by the day, and he and his brother seem to always want to be together, even when they struggle to maintain domestic harmony. But, being alone is boring. The cats agree!